Ch 20: WBCs Flashcards Preview

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Flashcards in Ch 20: WBCs Deck (39):
1

Neutrophil count that defines neutropenia? (List ranges for mild, moderate, and severe)

Mild: 1000-1500/ µL Moderate: 500-1000/ µL Severe: <500/ µL

2

What are the general principles that lead to neutropenia?

Decreased production, ineffective production, or increased destruction

3

List some causes of decreased production of neutrophils

Irradiation, drug induced, viral infection, congenital (Kostmann syndrome or infantile genetic agranulocytosis), cyclic

4

List causes of ineffective production of neutrophils

Megaloblastic anemia, myelodysplastic syndromes

5

List causes of increased destruction of neutrophils

isoimmune neonatal, autoimmune, idiopathic, drug induced, Felty syndrome, SLE, dialysis, splenic sequestration, increased margination **BIG PICTURE: 1) too much consumption, like in infection 2) too much sequestration, like in hypersplenism 3) too much Ab destruction

6

Neutrophil count that defines neutrophilia?

Above 7000/ µL

7

Causes of neutrophilia?

1) increased mobilization from bone marrow 2) enhanced release from marginal pool 3) stimulation of granulopoiesis

8

Huge elevation of WBCs, usually in response to acute infections or severe hemorrhage. May be mistaken for leukemia, esp CML. Distinguished by segmented neutrophils, high leukocyte alkaline phosphatase activity, and blue cytoplasmic inclusions (Dohle bodies).

LEUKEMOID REACTION

9

Seen in response to allergic reactions, parasitic infections, or persistent Ag-Ab complexes. Leads to necrosis, especially of myocardium and nervous system.

Eosinophilia - remember major basic protein causes the cell injury!

10

Commonly observed in immediate-type hypersensitivity reactions and chronic myeloproliferative neoplasms. Think histamine, chondroitin sulfate, leukotrienes.

Basophilia

11

Often occurs in neutropenic states as a compensatory mechanism

Monocytosis

12

Myeloproliferative neoplasm, derived from abnormal pluripotent stem cell, strong association with Philadelphia chromosome, BCR/ABL, t(9;22)

Chronic Myelogenous Leukemia

13

Phase of CML associated with lower blasts, normal platelets, hypercellularity in BM, and clusters of megakaryocytes.

Chronic phase

14

Phase of CML associated with increasing WBC count, increasing splenomegaly, persistent thrombocytopenia, and 10-20% blasts

Accelerated phase

15

Phase of CML associated with at least 20% blasts, extramedullary proliferation of blasts, clusters of blasts (hint: ___ from the past)

Blast phase

16

Myeloproliferative neoplasm from clonal hematopoietic stem cell that leads to autonomous production of RBCs not regulated by EPO. Associated with JAK2 mutation. Hyperplasia of all hematopoietic elements.

Polycythemia Vera

17

List the 3 stages of PV, and the erythropoietic pattern for each

1) Prepolycythemic - erythroid precursors abundant, maturation of cells normal 2) Polycythemic - erythroid precursors abundant, maturation of cells normal 3) Postpolycythemic - erythropoiesis ceases and there is an increase in extramedullary hematopoiesis (spleen!). Despite this EMH, stage characterized by anemia.

18

So PV is an unregulated increase of RBC production. How high can the lab values actually go?

Hemoglobin can exceed 20 g/dL. Hematocrit surpasses 60%. Whoa. This implies all kinds of circulation difficulties including angina pectoris, claudication, GI ulcers, thrombosis (stroke, MI)

19

Myeloproliferative neoplasm characterized by megakaryopoiesis, granulopoiesis, and marrow fibrosis

Primary myelofibrosis

20

Stage of primary myelofibrosis presenting with thrombocytosis, hypercellular bone marrow, and megakaryocytes with atypical "cloud-like" nuclei

Prefibrotic (early stage)

21

Stage of primary myelofibrosis presenting with tear drop RBCs, a characteristic reticulin or collagen fibrosis in the marrow, and extramedullary hematopoiesis

Fibrotic (late stage)

22

Myeloproliferative neoplasm associated with JAK2 mutation or MPL mutation. Peak incidence ~70yo. In rare cases, can transform to AML.

Primary myelofibrosis

23

What do the megakaryocytes distinctly look like in essential thrombocytopenia?

Stag-horn-shaped  

24

Uncontrolled proliferation of megakaryocytes, accompanied by recurrent episodes of thrombosis and hemorrhage?

Essential thrombocytopenia

25

Abnormal accumulation of mast cells in tissues

Mastocytosis

26

Form of mastocytosis associated with nodules on skin, usually occurring in kids that can spontaneously resolve

Cutaneous - most common is urticaria pigmentosusm

27

Form of mastocytosis associated with infiltration of mast cells to various organs. Can be indolent, aggressive, or leukemic. Often due to c-kit mutation, and compact aggregates of mast cells usually form

Systemic

28

Group of syndromes characterized by peripheral blood cytopenia, marrow failure, and dysplastic morphology in one or more blood cell lineage

Myelodysplastic syndromes (up to 40% of these patients progress to have AML)

29

All subtypes of myelodysplastic syndromes manifest these

Refractory anemia and/or cytopenia.

No erythrocytosis, leukocytosis, or thrombocytosis. 

30

Risk factors for developing a myelodysplastic syndrome

Hx of tx with alkylating agent or topo II inhibitor, radiation, benzene exposure, cigarette smoking, some congenital disorders

31

Clonal proliferation of myeloblasts in the marrow, MPO+, may have presence of Auer rods in cytoplasm?

Acute Myeloid Leukemia

32

What are the various classifications of AML?

1) Recurrent genetic abnormalities, 2) myelo-dysplasia related changes, 3) therapy related, 4) not otherwise categorized, 5) myeloid sarcoma, 6) related to Down Syndrome

33

Type of AML with genetic abnormality t(15;17) that results in PML/RAR-alpha fusion gene. Patients frequently present with DIC. 

Acute promyelocytic leukemia

34

Extramedullary solid tumor of myeloblasts or monoblasts (called chloroma, granulocytic sarcoma, or monoblastic sarcome)

Myeloid sarcoma

35

Pt presents with headache, itching, and splenomegaly. High Hgb (19.8), high WBCs (12,800), high platelets (550,000). Bone marrow displays hypercellularity. What kind of myeloproliferative disorder do they most likely have?

Polycythemia vera- uncontrolled upregulation of RBCs. Look for symptoms of hyperviscosity of blood (headache, blurry vision, itching, thrombosis/cerebrovascular accident) 

36

An older pt presents with increasing fatigue and pancytopenia. BM shows hypercellularity and ringed sideroblasts. What is the likely diagnosis? What is the best explanation of pathogenesis?

Myelodysplastic syndrome - changes in more than one cell lineage, can display ringed sideroblasts.

Usually occurs due to a clonal stem cell defect.

37

60 yo pt presents with night sweats, weight loss, fatigability, discomfort in abdominal ULQ. Labs show marked leukocytosis, with a lot of mature granulocytes, myelocytes, and basophils. BM is hypercellular with mostly WBC precursors. What is your best diagnosis?

Chronic myelogenous leukemia. Forgot to mention that they found a t(9;22), with abl at the chromosomal breakpoint. 

38

60 yo pt presents with weakness, hematuria, hepatosplenomegaly, and numerous ecchymoses. Normochromic normocytic anemia, thrombocytopenia, neutropenia, and marked leukocytosis with myeloblasts in the peripheral blood are present. What is going on?

Pt has AML, which suppresses normal hematopoiesis

39

48 yo pt presents with productive cough and fever. Leukocytosis and elevated leukocyte alkaline phosphatase are seen in labs. Is this CML, or a leukemoid reaction? How did you differentiate?

Leukemoid reaction! 1) LAP is elevated 

Other things to look for are comparison of maturity of cells in peripheral blood vs marrow, blue cytoplasmic inclusions called Dohle bodies, and comparison of basophils